Title : Comprehensive symptom management and supportive nursing care in a preterm toddler undergoing HSCT for pyruvate kinase deficiency
Abstract:
Background: Preterm toddlers undergoing hematopoietic stem cell transplantation (HSCT) face heightened vulnerability to treatment-related toxicities, infectious complications, and challenges in medication tolerance. Pyruvate kinase deficiency (PKD) further exacerbates anemia, transfusion dependence, and infection risk. While nursing plays a central role in pediatric HSCT, reports detailing comprehensive nursing strategies for high-risk preterm children remain limited.
Methods: A descriptive case study was conducted on a 2.5-year-old preterm boy (36 weeks, 1.9 kg) with transfusion-dependent PKD undergoing allogeneic HSCT from a fully HLA-matched sibling after Busulfan-Cyclophosphamide-Thiotepa-Fludarabine conditioning. Nursing care constituted the core of clinical management and included: (1) proactive symptom surveillance with rapid nursing-initiated responses to neutropenic fever; (2) structured mucositis and pain management through intensive oral care, multimodal analgesia, and hydration support; (3) tailored medication and nutritional strategies to address poor oral tolerance; (4) stringent central venous catheter protocols to prevent catheter-related bloodstream infections; and (5) family-centered education enabling safe line care, infection monitoring, medication administration, and home follow-up.
Results: Despite severe mucositis, feeding intolerance, and neutropenic fever, the patient developed no major infections or organ toxicities. Platelet engraftment was achieved on Day +14 and neutrophil recovery on Day +15. Full donor chimerism (100%) was confirmed by Day +28 and remained stable through Day +370. Nursing-led interventions were essential in preventing complications, ensuring treatment adherence, and facilitating safe discharge and home care.
Conclusions: This case emphasizes the critical role of specialized nursing in managing preterm toddlers undergoing HSCT for PKD. Proactive symptom monitoring, rigorous infection prevention, individualized medication and nutritional support, and structured caregiver training are key contributors to safe and successful transplant outcomes.
Keywords: hematopoietic stem cell transplantation, pyruvate kinase deficiency, pediatric nursing, preterm toddler, symptom management, infection prevention.
